New Therapeutic Approaches in Polycythemia Vera

Falchi, Lorenzo; Newberry, Kate J.; Verstovšek, Srđan

doi:10.1016/j.clml.2015.02.013

Cited by 12 publications

(12 citation statements)

References 75 publications

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“…Interferons have been used in treatment of MPNs, including PV, for almost 40 years, but the high prevalence of side effects have limited their clinical application 15. The novel pegylated form of interferon has advantages of longer half-life, better stability, less immunogenicity and improved tolerance 16.…”

Section: Discussionmentioning

confidence: 99%

Ruxolitinib treatment in an infant with JAK2+ polycythaemia vera-associated Budd-Chiari syndrome

Coşkun¹,

Height

Dhawan

et al. 2017

BMJ Case Reports

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Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction commonly seen with myeloproliferative neoplasms (MPNs). Polycythaemia vera (PV) is a very rare MPN in childhood. This is the youngest reported patient diagnosed with PV and BCS secondary to mutation.A 26-month-old girl was admitted with a 5-month history of abdominal distension, hepatosplenomegaly and ascites. Imaging studies revealed occlusion of the right hepatic vein and marked attenuation of the middle and left hepatic veins. BCS was diagnosed after excluding other causes of chronic liver disease. Mandatory prothrombotic workup revealed underlying PV.Partial recanalisation of hepatic veins occurred following anticoagulation therapy and PV was well controlled by pegylated interferon and hydroxycarbamide until she developed nephrotic syndrome, likely secondary to pegylated interferon. Therefore, treatment was modified to ruxolitinib, a novel-JAK-2 inhibitor; the therapy has been effective for almost 20 months with a good response and has no side effects.

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Section: Discussionmentioning

confidence: 99%

Ruxolitinib treatment in an infant with JAK2+ polycythaemia vera-associated Budd-Chiari syndrome

Coşkun¹,

Height

Dhawan

et al. 2017

BMJ Case Reports

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“…While the discovery of the Philadelphia chromosome as genetic alteration in CML and the arrival of BCR-ABL targeting TKIs in the clinic have largely replaced the need for IFNα-based treatment in this disease, some recent clinical studies have shown that combination of imatinib and IFNα is superior to either therapy alone, perhaps due to the fact that IFNα targets preferentially CML stem cells (Talpaz et al, 2013 ). Notably, due to its success in numerous clinical trials, IFNα is used as first-line treatment choice for patients with high-risk PV (Barbui et al, 2011 ; Falchi et al, 2015 ). Regardless of the specific use, IFN-based therapies have their limitations due to dose-limiting side effects.…”

Section: Prevention and Therapeutic Interventionmentioning

confidence: 99%

Underlying Causes and Therapeutic Targeting of the Inflammatory Tumor Microenvironment

Comen

Bowman

Kleppe

2018

Front. Cell Dev. Biol.

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Historically, the link between chronic inflammation and cancer has long been speculated. Only more recently, pre-clinical and epidemiologic data as well as clinical evidence all point to the role of the tumor microenvironment as inextricably connected to the neoplastic process. The tumor microenvironment (TME), a complex mix of vasculature, inflammatory cells, and stromal cells is the essential “soil” helping to modulate tumor potential. Increasingly, evidence suggests that chronic inflammation modifies the tumor microenvironment, via a host of mechanisms, including the production of cytokines, pro-inflammatory mediators, angiogenesis, and tissue remodeling. Inflammation can be triggered by a variety of different pressures, such as carcinogen exposure, immune dysfunction, dietary habits, and obesity, as well as genetic alterations leading to oncogene activation or loss of tumor suppressors. In this review, we examine the concept of the tumor microenvironment as related to both extrinsic and intrinsic stimuli that promote chronic inflammation and in turn tumorigenesis. Understanding the common pathways inherent in an inflammatory response and the tumor microenvironment may shed light on new therapies for both primary and metastatic disease. The concept of personalized medicine has pushed the field of oncology to drill down on the genetic changes of a cancer, in the hopes of identifying individually targeted agents. Given the complexities of the tumor microenvironment, it is clear that effective oncologic therapies will necessitate targeting not only the cancer cells, but their dynamic relationship to the tumor microenvironment as well.

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“…La PV puede permanecer clínicamente silenciosa, el 40% de los pacientes relatan síntomas debilitantes. Las manifestaciones clínicas de la enfermedad incluyen síntomas constitucionales (fatiga, prurito y sudores nocturnos), síntomas microvasculares (cefalea, aturdimiento, parestesias acrales, eritromelalgia -síndrome clínico caracterizado por con aumento de la temperatura de la piel y dolor de carácter urente localizado en las extremidades-así como dolor torácico atípico), y complicaciones macrovasculares (trombosis venosa profunda, tromboembolismo pulmonar, accidentes cerebrovasculares, angina inestable e infarto agudo de miocardio) [22]. Aproximadamente el 70% de los pacientes refieren la presencia de prurito acuagénico, que es aquel que es provocado por el contacto con agua.…”

Section: Presentación Clínica Y Diagnósticounclassified

Policitemia vera: presentación clínica, diagnóstico y nuevos abordajes terapéuticos.

Saavedra

Mejía

2018

archmed

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Introducción: Los desórdenes mieloproliferativos como la Policitemia Vera (PV) pertenecientes al grupo clásico filadelfia negativo, son un área hematología que requiere un acercamiento de mayor profundidad, de modo que sea posible ofrecer un abordaje clínico, diagnóstico y terapéutico de carácter amplio, pertinente y actualizado. Así será factible ofrecer alternativas a individuos que no responden a un tratamiento convencional.Objetivo: Recoger los elementos clínicos característicos o de mayor importancia de la Policitemia vera y realizar una contextualización sobre las opciones terapéuticas existentes o disponibles y las nuevas alternativas en desarrollo, desde diferentes disciplinas médicas básicas como la biología molecular y el área de la inmunología.Materiales y métodos: Se realizó durante el segundo semestre de 2017 y el primer trimestre de 2018 una revisión exhaustiva de material bibliográfico existente sobre la Policitemia vera, su fisiopatología, enfoque terapéutico actual y las nuevas alternativas médicas disponibles para su tratamiento, todo lo anterior en la base de datos de la Universidad Pontificia Bolivariana, utilizando motores de búsqueda como lo son PubMed, Clinical Key, SciELO y Science Direct.Conclusión: Es de vital importancia el estudio y la comprensión de la fisiopatología de la Policitemia vera para llegar, en un futuro, a la instauración de nuevas alternativas terapéuticas para los pacientes que la padecen y no responden a terapias tradicionales, pues aún no existe cura para esta condición hematológica y actualmente su enfoque terapéutico se centra principalmente, en controlar, mitigar y prevenir las complicaciones asociadas a la misma.

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New Therapeutic Approaches in Polycythemia Vera

Cited by 12 publications

References 75 publications

Ruxolitinib treatment in an infant with JAK2+ polycythaemia vera-associated Budd-Chiari syndrome

Ruxolitinib treatment in an infant with JAK2+ polycythaemia vera-associated Budd-Chiari syndrome

Underlying Causes and Therapeutic Targeting of the Inflammatory Tumor Microenvironment

Policitemia vera: presentación clínica, diagnóstico y nuevos abordajes terapéuticos.

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